Spinal fusion, ending caudally at L5 rather than at the sacrum, is recommended for selected patients with scoliosis due to Duchenne muscular dystrophy. We present a retrospective review of 48 patients operated on for this condition. Patients having spinal curvature with a Cobb angle of less than 40° and with less than 10° between a line tangential to the superior margins of both iliac crests and a line perpendicular to the spinous processes of L4 and L5, were fused to L5 (38 patients); patients not meeting these criteria were fused to the sacrum (10 patients).

Spinal and sitting obliquity increased in patients fused to L5, rather than to the sacrum, but the severity of the worsening obliquity was significantly greater in patients in whom the apex of the curve was below L1. Two of the ten latter patients required revision procedures for worsening obliquity when their pulmonary function deteriorated to less than 25% of predicted values.

We recommend fusion to the sacrum for scoliosis in Duchenne muscular dystrophy, especially for patients with an apex to their curve below L1.

Aims. It is uncertain whether instrumented spinal fixation in nonambulatory children with neuromuscular scoliosis should finish at L5 or be extended to the pelvis. Pelvic fixation has been shown to be associated with up to 30% complication rates, but is regarded by some as the standard for correction of deformity in these conditions. The incidence of failure when comparing the most caudal level of instrumentation, either L5 or the pelvis, using all-pedicle screw instrumentation has not previously been reported. In this retrospective study, we compared nonambulatory patients undergoing surgery at two centres: one that routinely instrumented to L5 and the other to the pelvis. Methods. In all, 91 nonambulatory patients with neuromuscular scoliosis were included. All underwent surgery using bilateral, segmental, pedicle screw instrumentation. A total of 40 patients underwent fusion to L5 and 51 had their fixation extended to the pelvis. The two groups were assessed for differences in terms of clinical and radiological findings, as well as complications. Results. The main curve (MC) was a mean of 90° (40° to 141°) preoperatively and 46° (15° to 82°) at two-year follow-up in the L5 group, and 82° (33° to 116°) and 19° (1° to 60°) in the pelvic group (p < 0.001 at follow-up). Correction of MC and pelvic obliquity (POB) were statistically greater in the pelvic group (p < 0.001). There was no statistically significant difference in the operating time, blood loss, or complications. Loss of MC correction (> 10°) was more common in patients fixated to the pelvis (23% vs 3%; p = 0.032), while loss of pelvic obliquity correction was more frequent in the L5 group (25% vs 0%; p = 0.007). Risk factors for loss of correction (either POB or MC) included preoperative coronal imbalance (> 50 mm, odds ratio (OR) 11.5, 95%confidence interval (CI) 2.0 to 65; p = 0.006) and postoperative sagittal imbalance (> 25 mm, OR 11.0, 95% CI1.9 to 65; p = 0.008). Conclusion. We found that patients undergoing pelvic fixation had a greater correction of MC and POB. The rate of complications was not different. Preoperative coronal and postoperative sagittal imbalance were associated with increased risks of loss of correction, regardless of extent of fixation. Therefore, we recommend pelvic fixation in all nonambulatory children with neuromuscular scoliosis where coronal or sagittal imbalance are present preoperatively. Cite this article: Bone Joint J 2020;102-B(2):261–267

Aims. To report the outcome of spinal deformity correction through anterior spinal fusion in wheelchair-bound patients with myelomeningocele. Methods. We reviewed 12 consecutive patients (7M:5F; mean age 12.4 years (9.2 to 16.8)) including demographic details, spinopelvic parameters, surgical correction, and perioperative data. We assessed the impact of surgery on patient outcomes using the Spina Bifida Spine Questionnaire and a qualitative questionnaire. Results. The mean follow-up was 5.4 years (2 to 14.9). Nine patients had kyphoscoliosis, two lordoscoliosis, and one kyphosis. All patients had a thoracolumbar deformity. Mean scoliosis corrected from 89.6° (47° to 151°) to 46.5° (17° to 85°; p < 0.001). Mean kyphosis corrected from 79.5° (40° to 135°) to 49° (36° to 65°; p < 0.001). Mean pelvic obliquity corrected from 19.5° (8° to 46°) to 9.8° (0° to 20°; p < 0.001). Coronal and sagittal balance restored to normal. Complication rate was 58.3% (seven patients) with no neurological deficits, implant failure, or revision surgery. The degree of preoperative spinal deformity, especially kyphosis and lordosis, correlated with increased blood loss and prolonged hospital/intensive care unit stay. The patients reported improvement in function, physical appearance, and pain after surgery. The parents reported decrease in need for everyday care. Conclusion. Anterior spinal fusion achieved satisfactory deformity correction with high perioperative complication rates, but no long-term sequelae among children with high level myelomeningocele. This resulted in physical and functional improvement and high reported satisfaction. Cite this article: Bone Joint J 2021;103-B(6):1133–1141

Aims. To report the mid-term results of a modified self-growing rod (SGR) technique for the treatment of idiopathic and neuromuscular early-onset scoliosis (EOS). Methods. We carried out a retrospective analysis of 16 consecutive patients with EOS treated with an SGR construct at a single hospital between September 2008 and December 2014. General demographics and deformity variables (i.e. major Cobb angle, T1 to T12 length, T1 to S1 length, pelvic obliquity, shoulder obliquity, and C7 plumb line) were recorded preoperatively, and postoperatively at yearly follow-up. Complications and revision procedures were also recorded. Only patients with a minimum follow-up of five years after surgery were included. Results. A total of 16 patients were included. Six patients had an idiopathic EOS while ten patients had a neuromuscular or syndromic EOS (seven spinal muscular atrophy (SMA) and three with cerebral palsy or a syndrome). Their mean ages at surgery were 7.1 years (SD 2.2) and 13.3 years (SD 2.6) respectively at final follow-up. The mean preoperative Cobb angle of the major curve was 66.1° (SD 8.5°) and had improved to 25.5° (SD 9.9°) at final follow-up. The T1 to S1 length increased from 289.7 mm (SD 24.9) before surgery to 330.6 mm (SD 30.4) immediately after surgery. The mean T1 to S1 and T1 to T12 growth after surgery were 64.1 mm (SD 19.9) and 47.4 mm (SD 18.8), respectively, thus accounting for a mean T1 to S1 and T1 to T12 spinal growth after surgery of 10.5 mm/year (SD 3.7) and 7.8 mm/year (SD 3.3), respectively. A total of six patients (five idiopathic EOS, one cerebral palsy EOS) had broken rods during their growth spurt but were uneventfully revised with a fusion procedure. No other complications were noted. Conclusion. Our data show that SGR is a safe and effective technique for the treatment of EOS in nonambulatory hypotonic patients with a neuromuscular condition. Significant spinal growth can be expected after surgery and is comparable to other published techniques for EOS. While satisfactory correction of the deformity can be achieved and maintained with this technique, a high rate of rod breakage was seen in patients with an idiopathic or cerebral palsy EOS. Cite this article: Bone Joint J 2020;102-B(11):1560–1566

A consecutive series of 85 patients with Duchenne’s muscular dystrophy who underwent spinal fusion over a period of 16 years was followed up with regard to the progression of the scoliosis and pelvic obliquity. Of 74 patients with adequate radiographic follow-up, 55 were instrumented with the Luque single-unit rod system and 19 with the Isola pedicle screw system; seven were instrumented to L3/4, 42 to L5, 15 to S1 and 10 to the pelvis with intrailiac rods. The mean period of follow-up was 49 months (SD 22) before and 47 months (SD 24) after operation. There was one peri-operative death and three cases of failure of hardware. The mean improvement in the Cobb angle was 26° and in pelvic obliquity, 9.2°. Fusion to L3/4 achieved a poorer correction of both curves while intrapelvic rods, achieved and maintained the best correction of pelvic obliquity. Fusion to S1 did not provide any benefit over more proximal fusion excluding the sacrum, with regard to correction and maintenance of both angles. The Isola system appeared to provide and maintain a slightly better correction of the Cobb angle

Aim:. To present 11 patients with quadriplegia who developed severe lordoscoliosis or hyperlordosis. This is a rare deformity in children with CP, treatment is challenging and there are less than 20 patients ever reported. Method:. All patients underwent posterior spinal arthrodesis at mean age 14.6 years with mean follow-up 3.5 years. We measured all radiographic parameters including coronal and sagittal balance and sacral slope before and after surgery. Results:. Mean preoperative lumbar lordosis was 107°. This corrected to mean 63° at follow-up. Mean preoperative thoracic kyphosis was 13°. This improved to mean 47° at follow-up. Mean preoperative scoliosis was 80°. This corrected to mean 22o at follow up. Mean preoperative pelvic obliquity was 22°. This corrected to mean 4° at follow-up. Mean preoperative sacral slope was 80o. This corrected to mean 51o at follow-up. Mean preoperative coronal imbalance was 5.2 cm. This corrected to mean 0.6 cm at follow-up. Mean preoperative sagittal imbalance was 8 cm. This corrected to mean 1.6 cm at follow-up. Mean surgical time was 260 minutes. Mean intra-operative blood loss was 0.82 EBV. Mean stay in ICU was 3.6 and in hospital 15.2 days. Complications included 3 patients with severe blood loss (1.3–2 EBV), one patient with chest and one chest and urinary infection, and a patient with superior mesenteric artery syndrome. Increased preoperative lumbar lordosis and sacral slope correlated with surgical and postoperative morbidity. In contrast, there was no correlation between preoperative scoliosis or pelvic obliquity and surgical morbidity. Reduced lumbar lordosis and increased thoracic kyphosis correlated with better global sagittal balance at follow-up. Greater surgical time and blood loss correlated with increased postoperative morbidity. All 11 patients and their parents reported excellent feedback on the outcome of surgery with major improvement in physical appearance, sitting balance and relief of severe preoperative back pain. Discussion:. Lordoscoliosis and hyperlordosis are associated with significant morbidity in patients with quadriplegia. The sagittal imbalance is the major component of the deformity and this can be corrected satisfactorily through a posterior spinal arthrodesis which produced excellent functional results and increased patients'/caretakers' satisfaction. Conflict Of Interest Statement: No conflict of interest

Purpose of the study. To investigate the efficacy of pedicle screw instrumentation in correcting spinal deformity in patients with quadriplegic cerebral palsy. Also to assess quality of life and functional improvement after deformity correction as perceived by the parents of our patients. Summary of Background Data. All pedicle screw constructs have been commonly used to correct adolescent idiopathic scoliosis. There is limited information on their effectiveness in treating patients with cerebral palsy and neuromuscular scoliosis. Methods. We reviewed the medical records and serial radiographs of 45 consecutive patients with quadriplegia who underwent spinal arthrodesis using pedicle screw/rod instrumentation and a standardised surgical technique (prospectively collected single surgeon's series). All patients were wheelchair bound with collapsing thoracolumbar scoliosis and pelvic obliquity. Twenty-eight patients had associated sagittal deformities. A telephone survey was performed by an independent investigator to assess parents' perception on surgical outcome. Results. Thirty-eight patients underwent posterior-only and 7 staged anteroposterior spinal arthrodesis. Mean age at surgery was 13.4 years (range 9-18.3) and mean postoperative follow-up 3.5 years (range 2.8-5). Pedicle screw instrumentation extended from T2/T3 to L5 with bilateral pelvic fixation using iliac bolts. Scoliosis was corrected from mean 82.5° to 21.4° (74.1%). Pelvic obliquity was corrected from mean 24° to 4° (83.3%). In posterior-only procedures, average blood loss was 0.8 blood volumes, ICU stay 3.5 days, and hospital stay 17.6 days. In anteroposterior procedures, average blood loss was 0.9 blood volumes, ICU stay 8.9 days, and hospital stay 27.4 days. Major complications included one deep infection and one re-operation to remove prominent implants but no deaths, no neurological deficit and no detected pseudarthrosis. Parents' survey demonstrated 100% satisfaction rate. Conclusion. Pedicle screw instrumentation can achieve excellent correction of spinopelvic deformity in quadriplegic cerebral palsy with low complication and re-operation rates and high parent satisfaction. Our study has demonstrated that spinal correction using segmental pedicle screw/rod constructs can be performed safely and with lesser major complications and reoperations compared to the traditionally used Unit rod or hybrid instrumentation. The greater degree of deformity correction and lesser rate of complications and reoperations due to non-union, prominent instrumentation or failed pelvic fixation using a pedicle screw compared to the Unit rod technique should be balanced against the increased implant cost

We classified fixed pelvic obliquity in patients after poliomyelitis into two major types according to the level of the pelvis relative to the short leg. Each type was then divided into four subtypes according to the direction and severity of the scoliosis. In 46 patients with type-I deformity the pelvis was lower and in nine with type II it was higher on the short-leg side. Subtype-A deformity was a straight spine with a compensatory angulation at the lower lumbar level, mainly at L4-L5, subtype B was a mild scoliosis with the convexity to the short-leg side, subtype C was a mild scoliosis with the convexity opposite the short-leg side, and subtype D was a moderate to severe paralytic scoliosis with the convexity to the short-leg side in type I and to the opposite side in type II. A combination of surgical procedures improved the obliquity in most patients. These included lumbodorsal fasciotomy, abductor fasciotomy and stabilisation of the hip by triple innominate osteotomy with or without transiliac lengthening. In patients with type ID or type IID appropriate spinal fusion was usually necessary

We describe 13 patients with cerebral palsy and lordoscoliosis/hyperlordosis of the lumbar spine who underwent a posterior spinal fusion at a mean age of 14.5 years (10.8 to 17.4) to improve sitting posture and relieve pain. The mean follow-up was 3.3 years (2.2 to 6.2).

The mean pre-operative lumbar lordosis was 108^° (80 to 150^°) and was corrected to 62^° (43^° to 85^°); the mean thoracic kyphosis from 17^° (-23^° to 35^°) to 47^° (25^° to 65^°); the mean scoliosis from 82^° (0^° to 125^°) to 22^° (0^° to 40^°); the mean pelvic obliquity from 21^° (0^° to 38^°) to 3^° (0^° to 15^°); the mean sacral slope from 79^° (54^° to 90^°) to 50^° (31^° to 66^°). The mean pre-operative coronal imbalance was 5 cm (0 cm to 8.9 cm) and was corrected to 0.6 cm (0 to 3.2). The mean sagittal imbalance of -8 cm (-16 cm to 7.8 cm) was corrected to -1.6 cm (-4 cm to 2.5 cm). The mean operating time was 250 minutes (180 to 360 minutes) and intra-operative blood loss 0.8 of estimated blood volume (0.3 to 2 estimated blood volume). The mean intensive care and hospital stay were 3.5 days (2 to 8) and 14.5 days (10 to 27), respectively. Three patients lost a significant amount of blood intra-operatively and subsequently developed chest or urinary infections and superior mesenteric artery syndrome.

An increased pre-operative lumbar lordosis and sacral slope were associated with increased peri-operative morbidity: scoliosis and pelvic obliquity were not. A reduced lumbar lordosis and increased thoracic kyphosis correlated with better global sagittal balance at follow-up. All patients and their parents reported excellent surgical outcomes.

Lordoscoliosis and hyperlordosis are associated with significant morbidity in quadriplegic patients. They are rare deformities and their treatment is challenging. Sagittal imbalance is the major component: it can be corrected by posterior fusion of the spine with excellent functional results.

Cite this article: Bone Joint J 2014;96-B:800–6.

The purpose of this study was to evaluate the long-term outcome of adults with spina bifida cystica (SBC) who had been treated either operatively or non-operatively for scoliosis during childhood.

We reviewed 45 patients with a SBC scoliosis (Cobb angle ≥ 50º) who had been treated at one of two children’s hospitals between 1991 and 2007. Of these, 34 (75.6%) had been treated operatively and 11 (24.4%) non-operatively. After a mean follow-up of 14.1 years (standard deviation (sd) 4.3) clinical, radiological and health-related quality of life (HRQOL) outcomes were evaluated using the Spina Bifida Spine Questionnaire (SBSQ) and the 36-Item Short Form Health Survey (SF-36).

Although patients in the two groups were demographically similar, those who had undergone surgery had a larger mean Cobb angle (88.0º (sd 20.5; 50.0 to 122.0); versus 65.7º (sd 22.0; 51.0 to 115.0); p < 0.01) and a larger mean clavicle–rib intersection difference (12.3 mm; (sd 8.5; 1 to 37); versus 4.1 mm, (sd 5.9; 0 to 16); p = 0.01) than those treated non-operatively. Both groups were statistically similar at follow-up with respect to walking capacity, neurological motor level, sitting balance and health-related quality of life (HRQOL) outcomes.

Spinal fusion in SBC scoliosis corrects coronal deformity and stops progression of the curve but has no clear effect on HRQOL.

Cite this article: Bone Joint J 2014; 96-B:1244–51

We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

We report the incidence of and risk factors for complications after scoliosis surgery in patients with Duchenne muscular dystrophy (DMD) and compare them with those of other neuromuscular conditions.

We identified 110 (64 males, 46 females) consecutive patients with a neuromuscular disorder who underwent correction of the scoliosis at a mean age of 14 years (7 to 19) and had a minimum two-year follow-up. We recorded demographic and peri-operative data, including complications and re-operations.

There were 60 patients with cerebral palsy (54.5%) and 26 with DMD (23.6%). The overall complication rate was 22% (24 patients), the most common of which were deep wound infection (9, 8.1%), gastrointestinal complications (5, 4.5%) and hepatotoxicity (4, 3.6%). The complication rate was higher in patients with DMD (10/26, 38.5%) than in those with other neuromuscular conditions (14/84, 16.7% (p = 0.019). All hepatotoxicity occurred in patients with DMD (p = 0.003), who also had an increased rate of deep wound infection (19% vs 5%) (p = 0.033). In the DMD group, no peri-operative factors were significantly associated with the rate of overall complications or deep wound infection. Increased intra-operative blood loss was associated with hepatotoxicity (p = 0.036).

In our series, correction of a neuromuscular scoliosis had an acceptable rate of complications: patients with DMD had an increased overall rate compared with those with other neuromuscular conditions. These included deep wound infection and hepatotoxicity. Hepatotoxicity was unique to DMD patients, and we recommend peri-operative vigilance after correction of a scoliosis in this group.

Cite this article: Bone Joint J 2014; 96-B:943–9.

We determined the frequency, rate and extent of development of scoliosis (coronal plane deformity) in wheelchair-dependent patients with Duchenne muscular dystrophy (DMD) who were not receiving steroid treatment. We also assessed kyphosis and lordosis (sagittal plane deformity). The extent of scoliosis was assessed on sitting anteroposterior (AP) spinal radiographs in 88 consecutive non-ambulatory patients with DMD. Radiographs were studied from the time the patients became wheelchair-dependent until the time of spinal fusion, or the latest assessment if surgery was not undertaken. Progression was estimated using a longitudinal mixed-model regression analysis to handle repeated measurements.

Scoliosis ≥ 10° occurred in 85 of 88 patients (97%), ≥ 20° in 78 of 88 (89%) and ≥ 30° in 66 of 88 patients (75%). The fitted longitudinal model revealed that time in a wheelchair was a highly significant predictor of the magnitude of the curve, independent of the age of the patient (p <  0.001). Scoliosis developed in virtually all DMD patients not receiving steroids once they became wheelchair-dependent, and the degree of deformity deteriorated over time.

In general, scoliosis increased at a constant rate, beginning at the time of wheelchair-dependency (p < 0.001). In some there was no scoliosis for as long as three years after dependency, but scoliosis then developed and increased at a constant rate. Some patients showed a rapid increase in the rate of progression of the curve after a few years – the clinical phenomenon of a rapidly collapsing curve over a few months.

A sagittal plane kyphotic deformity was seen in 37 of 60 patients (62%) with appropriate radiographs, with 23 (38%) showing lumbar lordosis (16 (27%) abnormal and seven (11%) normal).

This study provides a baseline to assess the effects of steroids and other forms of treatment on the natural history of scoliosis in patients with DMD, and an approach to assessing spinal deformity in the coronal and sagittal planes in wheelchair-dependent patients with other neuromuscular disorders.

Cite this article: Bone Joint J 2014;96-B:100–5.

We compared the clinical, radiological and quality-of-life outcomes between hybrid and total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis. A matched comparison using prospectively collected data was undertaken. A total of 66 patients underwent posterior or anteroposterior correction and fusion with hybrid (n = 33, mean age at surgery 15.8 years (9.10 to 19.6)) or total pedicle screw instrumentation (n = 33, mean age 14.7 years (7.0 to 20.7)) with a minimum follow-up of two years. The major curve pre-operatively was a mean of 87° (sd 29, 25° to 141°) and 81° (sd 18, 47° to 116°) in the hybrid and total pedicle screw groups, respectively (p = 0.29) and at a minimum of two years it was 33° (sd 20; 2° to 87°) and 20° (sd 12; 1° to 55°), respectively (p = 0.0016). The mean correction of the major curve was 59% (41% to 88%) in the hybrid and 75% (43% to 99%) in the total pedicle screw groups at two-year follow-up (p = 0.0011). The mean operating time was 7.45 hours (sd 2.18) and 6.04 hours (sd 1.71) in the hybrid and total pedicle screw groups, respectively (p = 0.001), and the mean intra-operative blood loss was 3760 ml (sd 2790) and 1785 ml (sd 1110), respectively (p = 0.001).

Total pedicle screw instrumentation provided shorter operating times, less blood loss and better correction of the major curve compared with hybrid constructs in patients undergoing surgery for neuromuscular scoliosis.

Few studies have examined the order in which a spinal osteotomy and total hip replacement (THR) are to be performed for patients with ankylosing spondylitis. We have retrospectively reviewed 28 consecutive patients with ankylosing spondylitis who underwent both a spinal osteotomy and a THR from September 2004 to November 2012. In the cohort 22 patients had a spinal osteotomy before a THR (group 1), and six patients had a THR before a spinal osteotomy (group 2). The mean duration of follow-up was 3.5 years (2 to 9). The spinal sagittal Cobb angle of the vertebral osteotomy segment was corrected from a pre-operative kyphosis angle of 32.4 (SD 15.5°) to a post-operative lordosis 29.6 (SD 11.2°) (p < 0.001). Significant improvements in pain, function and range of movement were observed following THR. In group 2, two of six patients had an early anterior dislocation. The spinal osteotomy was performed two weeks after the THR. At follow-up, no hip has required revision in either group. Although this non-comparative study only involved a small number of patients, given our experience, we believe a spinal osteotomy should be performed prior to a THR, unless the deformity is so severe that the procedure cannot be performed.

Cite this article: Bone Joint J 2014;96-B:360–5.